Sporadic inclusion body myositis: a continuing puzzle

  title={Sporadic inclusion body myositis: a continuing puzzle},
  author={Merrilee Needham and Frank L Mastaglia},
  journal={Neuromuscular Disorders},

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Inclusion body myositis: new insights into pathogenesis
Understanding the paradoxes in sporadic inclusion body myositis is important in determining rational therapy for the disease.
Inclusion body myositis: old and new concepts
Inclusion body myositis (IBM) is the most common idiopathic inflammatory myopathy occurring in patients over the age of 50 years and probably accounts for about 30% of all inflammatory myopathies.
Sporadic inclusion body myositis: new insights and potential therapy.
Despite improved knowledge, IBM continues to be a puzzling disease and the pathogenesis remains to be clarified and an interdisciplinary, bench to bedside translational research approach is crucial for the successful identification of novel treatments for this debilitating, currently untreatable disorder.
Sporadic inclusion body myositis: an unsolved mystery.
It remains uncertain whether sIBM is primarily an immune-mediated inflammatory myopathy or a degenerative myopathy with an associated inflammatory component.
Myouclear Breakdown in Sporadic Inclusion Body Myositis
Figures that show abnormal localization of nuclear proteins associated with MAP kinase in s-IBM muscle fibers suggest that inhibition of nuclear transport during myogenesis is suggested and the theory that nuclear damage is closely associated with its etiology is supported.
Sporadic inclusion body myositis: variability in prevalence and phenotype and influence of the MHC.
  • F. Mastaglia
  • Biology, Medicine
    Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology
  • 2009
Recent observations indicate that the clinical phenotype at presentation is also quite variable and that the influence of the MHC is more complex than previously appreciated with HLA alleles also having modifying effects on the age-at-onset, severity and rate of progression of the disease.
Vacuolar myopathy in a dog resembling human sporadic inclusion body myositis
Observations constitute the first evidence that both the inflammatory and degenerative features of human sIBM can occur in a non-human species.
Inß ammatory muscle diseases
Patients with DM or PM usually respond to treatment with glucocorticoids and immunosuppressive agents but their use remains largely empirical, and emerging therapies for resistant cases include TNFα inhibitors (etanercept, ins iximab) and monoclonal antibodies (rituximab, alemtuzumab).
Advances in inclusion body myositis: genetics, pathogenesis and clinical aspects
Understanding the aetiopathogeneis is vital to identify future treatment targets, and understanding the natural history and the roles of biomarkers including the anti-CN1a antibody is vital for designing future clinical trials in IBM.
Ongoing Developments in Sporadic Inclusion Body Myositis
Sporadic inclusion body myositis (IBM) is an acquired muscle disorder associated with ageing, for which there is no effective treatment. Ongoing developments include: genetic studies that may provide


Genetics of inclusion‐body myositis
Current understanding of the contribution of genetic susceptibility factors to the development of sIBM is summarized in this review.
Sporadic inclusion body myositis—diagnosis, pathogenesis and therapeutic strategies
  • M. Dalakas
  • Biology, Medicine
    Nature Clinical Practice Neurology
  • 2006
Emerging data imply that continuous upregulation of cytokines and major histocompatibility complex class I on the muscle fibers causes an endoplasmic reticulum stress response, resulting in intracellular accumulation of misfolded glycoproteins and activation of the transcription factor NFκB, leading to further cytokine activation.
Inclusion-body myositis
It is proposed that the identified abnormal accumulation, misfolding, and aggregation of proteins, perhaps provoked by the aging milieu and aggravated by the oxidative stress, lead to the s-IBM-specific vacuolar degeneration and atrophy of muscle fibers.
HLA associations with inclusion body myositis
The phenotype and phenotype data provide support for an autoimmune etiology for, and genetic predisposition to, IBM.
Inclusion body myositis in HIV-1 and HTLV-1 infected patients.
It is concluded that IBM occurs in HIV-1 and HTLV-1 infected individuals and has a clinical, histological and immunological pattern identical to sporadic IBM in the non-retrovirally infected patients.
Molecular pathology and pathogenesis of inclusion‐body myositis
The basic hypothesis is that overexpression of AβPP within the aging muscle fibers is an early upstream event causing a subsequent pathogenic cascade that leads to sporadic inclusion‐body myositis.
Proteomic Analysis of Inclusion Body Myositis
Proteomics is validates proteomics as a powerful tool in the study of muscle disease and indicates a unique pattern of protein expression in IBM, which mainly serve as carriers for muscle contraction and other normal muscle functions.
Inclusion body myositis: Investigation of the mumps virus hypothesis by polymerase chain reaction
The results therefore do not support the mumps hypothesis for IBM and the integrity of muscle RNA extracts was confirmed by PCR detection of constitutive Ableson tyrosine kinase mRNA.
Inclusion Body Myositis: Clonal Expansions of Muscle‐Infiltrating T Cells Persist Over Time
Results show that in IBM there is clonal restriction of TCR expression in muscle‐infiltrating lymphocytes, and this indicates an important, continuous, antigen‐driven inflammatory reaction in IBM.
Inclusion body myositis-like phenotype induced by transgenic overexpression of βAPP in skeletal muscle
Transgenic mice were derived in which selective overexpression of βAPP leads to the development of a subset of other histopathological and clinical features characteristic of IBM, including centric nuclei, inflammation, and deficiencies in motor performance, consistent with a pathogenic role for βAPP mismetabolism in human IBM.